The study is the first to outline how to limit and repair DNA damage defects in cells and could provide a model for understanding processes that cause us to age.
The findings could have significant benefits, such as reducing degeneration of some tissues in older age, and could assist health management in countries, including the UK, where average life expectancy is extending, according to the researchers.
The first results of the 18-month study, led by Durham University, are published in the journal Human Molecular Genetics.
Researchers looked at a group of inherited degenerative disorders called Laminopathies that are caused by mutations in the gene LMNA. The most severe disorders linked to mutation in this gene include Hutchinson Gilford Progeria Syndrome (HGPS), a fatal disease that causes premature ageing in children.
The Durham University and University of Bologna team used in-vitro models and molecular imaging techniques to measure levels of oxidative stress and DNA damage in cells. Oxidative stress relates to the dynamics of cells and the body's ability to detoxify and repair itself. When cells are stressed, levels of highly reactive molecules known as reactive oxygen species (ROS) can increase dramatically. This can result in significant damage to cell structures and to DNA which is one underlying cause of premature ageing and standard ageing.
The team monitored changes in thousands of 'crinkly', damaged cells after administering NAC, a widely-used and well-tolerated drug. They found that while this drug did not affect some aspects of cell stress that are effectively controlled by currently used drugs, it very effectively controlled ROS generation and DNA damage. The results suggest that administration of NAC in combination with currently used drugs might improve the health of children with progeria.
Professor Chris Hutchison, a member of the Biophysical Sciences Institute, Durham University, said: "In children with progeria, we can see that double-strand breaks in the DNA architecture of cells increase which in turn adds to poor rates of cell growth. Our treatment of these cells with the drug N-acetyl cysteine (NAC) reversed both of these effects.
"Mutations in the LMNA gene cause more diseases, such as muscular dystrophy, than any other that we know. We've found that DNA damage can be controlled and our findings could be an important step to helping both children with progeria and older people to live lives that are less debilitating in terms of health problems."
The researchers said their findings were at an early stage and further studies and human clinical trials would be needed to develop effective drug treatments.
Professor Hutchison added: "We are using a careful approach that will look at patients with progeria to see if there's a model that can be used for wider medicine. It would be great to find a way to help relieve some of the effects of progeria and to extend the children's lives, whilst also finding a way to help increasingly ageing populations in many parts of the world.
"The findings are at a very early stage but they show the potential for helping people to live more comfortable and less painful lives when they reach 70 and 80 years of age and beyond."
Hutchinson-Gilford Progeria Syndrome "Progeria" or "HGPS" is a rare, fatal genetic condition characterized by an appearance of accelerated aging in children. Progeria has a reported incidence of about 1 in 4 - 8 million newborns from all over the world. It affects both sexes equally and all races. Although they are born looking healthy, children with Progeria begin to display many characteristics of accelerated aging at around 18-24 months of age.
Progeria signs include growth failure, loss of body fat and hair, aged-looking skin, stiffness of joints, hip dislocation, generalized atherosclerosis, cardiovascular (heart) disease and stroke. The children have a remarkably similar appearance, despite differing ethnic backgrounds. Children with Progeria die of atherosclerosis (heart disease) at an average age of thirteen years (with a range of about 8 – 21 years).
Dr. Leslie Gordon, Medical Director for The Progeria Research Foundation, said: "Dr. Hutchison's study has not only confirmed basic cellular defects in Progeria, but has also identified potential ways to improve those defects. This type of biological science is how progress towards treatments and a cure for children with Progeria will advance."
The research could also provide a model for the future for tailoring treatments and dosages of drugs to the individual and therefore improving patient health where drugs are administered.
The project was funded by the Association for Cancer Research, One North East and EU FP6.
Carl Stiansen | EurekAlert!
The importance of biodiversity in forests could increase due to climate change
17.11.2017 | Deutsches Zentrum für integrative Biodiversitätsforschung (iDiv) Halle-Jena-Leipzig
Win-win strategies for climate and food security
02.10.2017 | International Institute for Applied Systems Analysis (IIASA)
The WHO reports an estimated 429,000 malaria deaths each year. The disease mostly affects tropical and subtropical regions and in particular the African continent. The Fraunhofer Institute for Silicate Research ISC teamed up with the Fraunhofer Institute for Molecular Biology and Applied Ecology IME and the Institute of Tropical Medicine at the University of Tübingen for a new test method to detect malaria parasites in blood. The idea of the research project “NanoFRET” is to develop a highly sensitive and reliable rapid diagnostic test so that patient treatment can begin as early as possible.
Malaria is caused by parasites transmitted by mosquito bite. The most dangerous form of malaria is malaria tropica. Left untreated, it is fatal in most cases....
The formation of stars in distant galaxies is still largely unexplored. For the first time, astron-omers at the University of Geneva have now been able to closely observe a star system six billion light-years away. In doing so, they are confirming earlier simulations made by the University of Zurich. One special effect is made possible by the multiple reflections of images that run through the cosmos like a snake.
Today, astronomers have a pretty accurate idea of how stars were formed in the recent cosmic past. But do these laws also apply to older galaxies? For around a...
Just because someone is smart and well-motivated doesn't mean he or she can learn the visual skills needed to excel at tasks like matching fingerprints, interpreting medical X-rays, keeping track of aircraft on radar displays or forensic face matching.
That is the implication of a new study which shows for the first time that there is a broad range of differences in people's visual ability and that these...
Computer Tomography (CT) is a standard procedure in hospitals, but so far, the technology has not been suitable for imaging extremely small objects. In PNAS, a team from the Technical University of Munich (TUM) describes a Nano-CT device that creates three-dimensional x-ray images at resolutions up to 100 nanometers. The first test application: Together with colleagues from the University of Kassel and Helmholtz-Zentrum Geesthacht the researchers analyzed the locomotory system of a velvet worm.
During a CT analysis, the object under investigation is x-rayed and a detector measures the respective amount of radiation absorbed from various angles....
The quantum world is fragile; error correction codes are needed to protect the information stored in a quantum object from the deteriorating effects of noise. Quantum physicists in Innsbruck have developed a protocol to pass quantum information between differently encoded building blocks of a future quantum computer, such as processors and memories. Scientists may use this protocol in the future to build a data bus for quantum computers. The researchers have published their work in the journal Nature Communications.
Future quantum computers will be able to solve problems where conventional computers fail today. We are still far away from any large-scale implementation,...
15.11.2017 | Event News
15.11.2017 | Event News
30.10.2017 | Event News
21.11.2017 | Physics and Astronomy
21.11.2017 | Materials Sciences
21.11.2017 | Health and Medicine